vesicoureteral reflux and duplex systemsdownloads.hindawi.com/journals/au/2008/651891.pdfchild who...

Hindawi Publishing CorporationAdvances in UrologyVolume 2008, Article ID 651891, 3 pagesdoi:10.1155/2008/651891

Review ArticleVesicoureteral Reflux and Duplex Systems

John C. Thomas

Department of Urology, Division of Pediatric Urology, Monroe Carell Jr. Children’s Hospital at Vanderbilt,2200 Children’s Way, Nashville, TN 37232, USA

Correspondence should be addressed to John C. Thomas, [email protected]

Received 17 April 2008; Accepted 30 June 2008

Recommended by Walid A. Farhat

Vesicoureteral reflux (VUR) is the most common anomaly associated with duplex systems. In addition to an uncomplicated duplexsystem, reflux can also be secondary in the presence of an ectopic ureterocele with duplex systems. Controversy exists in regardto the initial and most definitive management of these anomalies when they coexist. This paper will highlight what is currentlyknown about duplex systems and VUR, and will attempt to provide evidence supporting the various surgical approaches to anectopic ureterocele and duplex system and the implications of concomitant VUR.

Copyright © 2008 John C. Thomas. This is an open access article distributed under the Creative Commons Attribution License,which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

1. INTRODUCTION

Less than 1% of the general population has a duplex kidney[1]. Females are affected more commonly than males and thisanomaly is bilateral in 17–33% of cases [2]. VUR is the mostcommon associated anomaly found in duplex kidneys andis present in 70% of these patients who present with a uri-nary tract infection [3, 4]. VUR almost always occurs intothe lower-pole moiety due to its lateral displacement withinthe bladder. If VUR is seen in the upper-pole moiety, onemust suspect a laterally displaced incomplete duplication oran ectopic orifice located within the bladder neck or urethra.This paper will review the natural history of VUR associatedwith uncomplicated duplex systems as well as the controver-sies that arise in managing reflux found in conjunction withectopic ureteroceles.

2. DISCUSSION

2.1. VUR and duplex systems

There are certain factors that contribute to reflux resolutionin single-system (SS) ureters, including patient age, grade ofreflux, postnatal presentation, and the presence or absenceof associated voiding dysfunction [4]. The natural history ofVUR in association with duplex systems (DSs) is not com-pletely clear. Despite several studies addressing this issue,

all were limited in some way by their noncontrolled retro-spective nature, patient selection or surgeon bias, and lim-ited long-term follow-up [4]. Lee et al. followed 1/3 of theirpatients with VUR and DS nonoperatively, and concludedthat resolution rates of low-grade (I-II/V) reflux were com-parable to those seen in SS [5]. Patients with high-grade re-flux were excluded from this study. A similar conclusion wasnoted in another study in which all grades of reflux were in-cluded. Spontaneous resolution occurred in over half of pa-tients with grades I–III/V VUR and support considerationfor initial conservative management with prophylactic an-tibiotics [6]. Over a two-year period of observation, Hus-mann et al. found that reflux resolved in 10% of patients withDS and grade II/V VUR as compared to 35% of a matchedgroup of patients with SS; however, there were no differencesin the incidence of breakthrough infections, additional renalscarring, or worsening reflux [7]. It seems clear that most pa-tients with DS and low grades (<III/V) can be initially man-aged conservatively; however, VUR will likely take longer toresolve as compared to SS VUR. Clinical information con-cerning high-grade VUR (IV-V) and DS is lacking, althoughone study documented no resolution at mean follow-up of 42months as well as an increased incidence of infectious com-plications, especially in young females [4].

Data from the available literature suggests that the major-ity of patients with DS and low-grade VUR can be initiallymanaged with antibiotics and careful observation. Parents

mailto:[email protected]

2 Advances in Urology

should be counseled that it may take longer for the refluxto resolve and young females with high-grade VUR maybe at increased risk for infections. Despite these findings,the absolute indication for surgery in individuals with low-grade VUR is not different from those with SS and simi-lar VUR, and surgical correction is successful in the ma-jority of cases [4]. In fact, one series reported a 98% suc-cess rate for common sheath reimplantation of uncompli-cated duplex systems, and concluded that the presence of aduplication anomaly does not adversely affect surgical out-come. Adequate tunnel width and long intravesical tunnelswere noted to be the most important technical aspects [8].It is important to remember, however, that complicated du-plex systems associated with the need for ureteroureteros-tomy, ureteral tapering or tailoring, or ureteropyelostomymay carry higher complication rates than uncomplicatedcommon sheath reimplantation.

2.2. Ectopic ureteroceles and vesicoureteral reflux

Duplex systems are an uncommon diagnosis causing prena-tal hydronephrosis; however, when confirmed, ureterocelesare one of the most common associated findings [9, 10]. Ec-topic ureteroceles can cause upper-pole hydronephrosis andobstruction, which leads to ipsilateral lower-pole reflux in50% of cases [11]. Contralateral reflux is seen in 25% of casesand reflux into the ureterocele occurs 10% of the time [12].

The initial and subsequent management of ureteroceleshas been controversial and depends on several factors, in-cluding presenting symptoms, ectopic versus orthotopic po-sition, presence or absence of reflux, and function of the as-sociated upper-pole moiety [11]. As the focus of this article isreflux and duplex systems, the discussion below will be lim-ited to the management of ectopic ureteroceles in patientswho present with concomitant reflux and a nonfunctioningor functioning upper-pole moiety. Management options in-clude endoscopic puncture and decompression, a simplifiedupper-tract approach, namely, heminephrectomy, or com-plete repair including upper-pole surgery, ureterocele exci-sion, and lower-tract reconstruction in a single setting.

In the above proposed setting, the clear indication forendoscopic decompression of an ectopic ureterocele is in achild who presents with sepsis or bladder outlet obstruction.However, in the setting of sepsis, one must open the urete-rocele completely, as puncturing may not result in adequatedrainage. This procedure almost invariably results in promptimprovement in patient symptoms, but the parents should becounseled that their child will require definitive reconstruc-tion at a later date, as reflux into the upper-pole moiety isthe rule, not the exception. In contrast, endoscopic punctureof an ectopic ureterocele in the nonemergent setting may alsocommit the patient to future reconstruction. In one series de-scribing endoscopic puncture for ectopic ureteroceles, Jayan-thi et al. reported postoperative reflux into the upper-polemoiety in 50% of cases [13]. Overall, 70% of their patientsunderwent open surgery with the vast majority at the levelof the bladder [13]. Some have argued that initial endoscopicdecompression may facilitate subsequent lower-tract surgeryby reducing the size of the upper-pole ureter [14].

Upper-pole heminephrectomy can result in excellent de-compression of the ureterocele and should be the procedureof choice if there is no ipsilateral lower pole or contralat-eral reflux [15]. Removing a functional upper pole has beenadvocated by some as this moiety only provides approxi-mately 15% of total renal function at best [16]. Alternatively,one can salvage the upper pole with a ureteroureterostomyor ureteropyelostomy and subtotal ureterectomy. Success ofthe upper-tract approach alone without the need for subse-quent bladder surgery is directly related to the presence orabsence of ipsilateral lower pole or contralateral reflux. Hus-mann et al. reported a definitive cure in only 16% of patientsin this setting if endoscopic decompression or an upper-tractapproach was used alone. In fact, the need for additionalsurgery was related to the number of renal moieties with re-flux at presentation, reporting a 96% reoperative rate withunilateral high-grade reflux or reflux seen in more than onerenal moiety [16].

In conclusion, ectopic ureteroceles that reflux or areassociated with reflux into other moieties are likely bestserved with ureterocele excision or marsupialization, bladderfloor reconstruction, and ureteral reimplant. Another optionwould be a ureteroureterostomy with a lower-pole extrav-esical reimplant. In those patients who present with sepsisor bladder outlet obstruction, endoscopic decompression ishighly successful but will likely commit the patient to fur-ther surgery. Upper-pole heminephrectomy is best appliedto those patients with nonfunctioning upper poles and noassociated reflux. In this setting, this approach is highly suc-cessful and has the advantage of avoiding bladder surgery,limiting risks to the lower pole, and eliminating the poten-tial unknown risks of preserving a dysplastic upper pole [15].Arguably, upper-pole heminephrectomy can be performedopen or laparoscopically.

3. CONCLUSIONS

Reflux found in association with a duplex system may takelonger to resolve than single-system reflux. Parents shouldbe counseled accordingly. Surgery to correct VUR in duplexsystems is highly successful. Ectopic ureteroceles can presentan interesting and difficult surgical challenge and can be ul-timately managed with multiple surgical approaches follow-ing initial conservative therapy. Endoscopic decompressionseems best reserved for the septic patient or one who presentswith bladder outlet obstruction. It provides excellent relief ofobstruction and can preserve upper-pole renal function. Ul-timately, these patients are currently managed by either anupper- or lower-tract approach. The most important factorin deciding which approach to take is the presence or absenceof VUR.

REFERENCES

[1] S. M. Whitten and D. T. Wilcox, “Duplex systems,” PrenatalDiagnosis, vol. 21, no. 11, pp. 952–957, 2001.

[2] W. E. Kaplan, P. Nasrallah, and L. R. King, “Reflux in completeduplication in children,” The Journal of Urology, vol. 120, no.2, pp. 220–222, 1978.

John C. Thomas 3

[3] J. T. J. Privett, W. D. Jeans, and J. Roylance, “The incidenceand importance of renal duplication,” Clinical Radiology, vol.27, no. 4, pp. 521–530, 1976.

[4] K. Afshar, F. Papanikolaou, R. Malek, D. Bagli, J. L. Pippi-Salle,and A. Khoury, “Vesicoureteral reflux and complete ureteralduplication. Conservative or surgical management?” The Jour-nal of Urology, vol. 173, no. 5, pp. 1725–1727, 2005.

[5] P. H. Lee, D. A. Diamond, P. G. Duffy, and P. G. Ransley, “Du-plex reflux: a study of 105 children,” The Journal of Urology,vol. 146, no. 2, part 2, pp. 657–659, 1991.

[6] D. S. Peppas, S. J. Skoog, D. A. Canning, and A. B. Belman,“Nonsurgical management of primary vesicoureteral reflux incomplete ureteral duplication: is it justified?” The Journal ofUrology, vol. 146, no. 6, pp. 1594–1595, 1991.

[7] D. A. Husmann and T. D. Allen, “Resolution of vesicoureteralreflux in completely duplicated systems: fact or fiction?” TheJournal of Urology, vol. 145, no. 5, pp. 1022–1023, 1991.

[8] P. I. Ellsworth, D. J. Lim, R. D. Walker, P. S. Stevens, M. A.Barraza, and H.-G. J. Mesrobian, “Common sheath reimplan-tation yields excellent results in the treatment of vesicoureteralreflux in duplicated collecting systems,” The Journal of Urology,vol. 155, no. 4, pp. 1407–1409, 1996.

[9] J. Mandell, B. R. Blythe, C. A. Peters, A. B. Retik, J. A. Estroff,and B. R. Benacerraf, “Structural genitourinary defects de-tected in utero,” Radiology, vol. 178, no. 1, pp. 193–196, 1991.

[10] L. D. Jee, A. M. K. Rickwood, M. P. L. Williams, P. A. M. Ander-son, and J. Mandell, “Experience with duplex system anoma-lies detected by prenatal ultrasonography,” The Journal of Urol-ogy, vol. 149, no. 4, pp. 808–810, 1993.

[11] D. E. Coplen and J. S. Barthold, “Controversies in the man-agement of ectopic ureteroceles,” Urology, vol. 56, no. 4,pp. 665–668, 2000.

[12] S. Sen, S. W. Beasley, S. Ahmed, and E. Durham Smith, “Renalfunction and vesicoureteric reflux in children with ureteroce-les,” Pediatric Surgery International, vol. 7, no. 3, pp. 192–194,1992.

[13] V. R. Jayanthi and S. A. Koff, “Long-term outcome of tran-surethral puncture of ectopic ureteroceles: initial success andlate problems,” The Journal of Urology, vol. 162, no. 3, part 2,pp. 1077–1080, 1999.

[14] C. S. Cooper, G. Passerini-Glazel, J. C. Hutcheson, et al.,“Long-term followup of endoscopic incision of ureteroceles:intravesical versus extravesical,” The Journal of Urology, vol.164, no. 3, part 2, pp. 1097–1100, 2000.

[15] D. Husmann, B. Strand, D. Ewalt, M. Clement, S. Kramer, andT. Allen, “Management of ectopic ureterocele associated withrenal duplication: a comparison of partial nephrectomy andendoscopic decompression,” The Journal of Urology, vol. 162,no. 4, pp. 1406–1409, 1999.

[16] M. A. Keating, “Ureteral duplication anomalies: ectopic ure-ters and ureteroceles,” in Clinical Pediatric Urology, S. G.Docimo, D. A. Canning, and A. E. Khoury, Eds., pp. 593–648,Informa Healthcare, London, UK, 2007.

Submit your manuscripts athttp://www.hindawi.com

Stem CellsInternational

Hindawi Publishing Corporationhttp://www.hindawi.com Volume 2014


MEDIATORSINFLAMMATION

of


Behavioural Neurology

EndocrinologyInternational Journal of



Disease Markers


BioMed Research International

OncologyJournal of



Oxidative Medicine and Cellular Longevity


PPAR Research

The Scientific World JournalHindawi Publishing Corporation http://www.hindawi.com Volume 2014

Immunology ResearchHindawi Publishing Corporationhttp://www.hindawi.com Volume 2014

Journal of

ObesityJournal of



Computational and Mathematical Methods in Medicine

OphthalmologyJournal of


Diabetes ResearchJournal of



Research and TreatmentAIDS


Gastroenterology Research and Practice


Parkinson’s Disease

Evidence-Based Complementary and Alternative Medicine

Volume 2014Hindawi Publishing Corporationhttp://www.hindawi.com

vesicoureteral reflux and duplex systemsdownloads.hindawi.com/journals/au/2008/651891.pdfchild who...

Documents